Surgical management in a severe OSA patient diagnosed with Stickler syndrome

Stickler syndrome is a genetic disorder of connective tissue. One of the major symptoms associated with this disorder is an oro-facial malformation, which may cause a submucous cleft or a complete cleft of the hard palate. A 32-year-old man diagnosed with Stickler syndrome and a submucosal cleft palate (SMCP) visited our hospital with a chief complaint of excessive daytime sleepiness. The patient was diagnosed with severe obstructive sleep apnea (OSA), and administration of a polysomnography test revealed an apnea-hypopnea index (AHI) of 30.9 events/hour (h). Auto-titrating continuous positive airway pressure was initiated to control the OSA symptoms and subsequently the patient showed some improvement. However, due to continuous velopharyngeal insufficiency symptoms, intravelar veloplasty was performed. Three months after surgery, the AHI had decreased to 12.4 events/h. Recent studies have described a greater risk for OSA in individuals with cleft palate, than in the general population. The present case demonstrates surgical success in a patient with OSA and SMCP, suggesting that palatal surgery may be considered an optional surgical treatment for OSA patients with SMCP.     

Evaluation of Prostate Cancer Stage Groups Updated in the 8th Edition of the American Joint Committee on Cancer Tumor–Node–Metastasis Staging Manual

Introduction

The American Joint Committee on Cancer (AJCC) tumor, node, metastasis classification system (TNM) staging manual has been updated and provides more specified stage grouping for prostate cancer (PCa). We aimed to validate the updated AJCC stage groups for PCa using a radical prostatectomy (RP) cohort.

Comprehensive Clinical and Genetic Characterization of Hyperprogression Based on Volumetry in Advanced Non–Small Cell Lung Cancer Treated With Immune Checkpoint Inhibitor

IntroductionHyperprogressive disease (HPD), characterized by accelerated tumor progression, has been proposed as a new pattern of progression after immune checkpoint inhibitor (ICI) treatment. The aim of this study was to describe the characteristics of HPD and investigate its predictive markers.MethodsClinical and radiological findings of 335 patients with advanced NSCLC treated with ICI monotherapy were retrospectively analyzed. Radiological data were quantitatively and longitudinally analyzed for tumor size and volume by comparing baseline and follow-up computerized tomography results. The findings were matched with individual genomic profiles generated by deep sequencing of 380 genes.ResultsAmong 135 patients with progressive disease (PD), as assessed by the Response Evaluation Criteria in Solid Tumors version 1.1 criteria, 48 (14.3% of all patients and 35.6% of those with PD) and 44 (13.1% of all patients and 32.6% among those with PD) were found to have HPD by volumetry (HPD_V) and assessed by RECIST 1.1 (HPD_R), respectively. Patients with HPD_V were associated with significantly inferior overall survival (OS) versus that of patients without HPD_V with PD (median OS = 4.7 months [95% confidence interval: 3.5–11.9)] versus 7.9 months [95% confidence interval: 6.0–13.5] [p = 0.004]); OS did not differ between patients without and without HPD_R. HPD_V status was an independent factor in OS. A derived neutrophil-to-lymphocyte ratio greater than 4 and lactate dehydrogenase level greater than the upper limit of normal were significantly associated with HPD_V. Moreover, we identified coinciding KRAS and serine/threonine kinase 11 gene (STK11) mutations in the cohort of patients with HPD_V (three of 16), whereas none were found in the cohort of patients without HPD_V (zero of 28).ConclusionsDefining HPD treated with ICI on the basis of volumetric measurement is more precise than is defining it on the basis of one-dimensional analysis. Pre-ICI derived neutrophil-to-lymphocyte ratio, lactate dehydrogenase level, and concurrence of STK11 and KRAS mutations could thus be used as potential biomarkers for HPD prediction.     

Persistent acidosis after reperfusion—A prognostic indicator of increased 30‐day and in‐hospital postoperative mortality in liver transplant recipients

During liver transplantation, the patient is at risk of developing progressive lactic acidosis. Following reperfusion, correction of acidosis may occur. In some patients, acidosis will worsen, a phenomenon referred to as persistent acidosis after reperfusion (PAAR). We compared postoperative outcomes in patients who manifested PAAR vs those that did not. All adult patients undergoing liver transplantation from 2002 to 2015 were included. PAAR is defined by the presence of a significant negative slope coefficient for base excess values measured after hepatic artery anastomosis through 72 hours postoperatively. Primary outcome was a composite of 30‐day and in‐hospital mortality. Secondary outcomes included: ICU LOS, total hospital LOS, and re‐transplantation rate within 7 days. PAAR occurred in 10% of the transplant recipients. Patients with PAAR had higher MELD, BMI, and eGFR and demonstrated a longer median ICU LOS and hospital median LOS with a trend toward mortality difference. But, after propensity matching, the mortality rate difference became significantly higher in patients with PAAR compared with matched controls while the ICU LOS differences disappeared. The re‐transplantation rates were similar also between the PAAR and no PAAR groups. The cohort with PAAR had a significant 30‐day and in‐hospital increase in mortality after propensity score matching.     

Real-World Experience with Etanercept Therapy and the Switching Pattern among Korean Patients with Psoriasis after Withdrawal of Etanercept

BackgroundThe efficacy and safety of etanercept in the treatment of psoriasis has been proven, and the drug was approved for the treatment of moderate to severe psoriasis. However, there have been few studies that have presented real-world data focused on concomitant treatment during etanercept treatment, and the switching pattern after discontinuation of etanercept.ObjectiveTo reveal the real-world treatment pattern of etanercept-based psoriasis treatment and to investigate the switching pattern after withdrawal of etanercept.MethodsWe enrolled 66 patients with psoriasis who were treated with etanercept. We collected data regarding the demographic characteristics of the patients, etanercept treatment schedules, and other treatments administered during the etanercept treatment period. We also investigated the treatment pattern after the discontinuation of etanercept with emphasis on the drug-free interval and the administered treatment modalities.ResultsThe mean treatment duration was 22.7±26.1 months and the mean number of etanercept injections was 21.5±27.9. Thirty-six patients were administered concomitant systemic medication or phototherapy. After discontinuation of etanercept, 54 patients were followed up and 34 of these patients were administered other systemic medication or phototherapy; phototherapy and cyclosporine was the most commonly administered treatment modality and 27.4% of treatments used biologics.ConclusionThe treatment schedule for etanercept was modified according to the severity of psoriasis and concomitant treatment was administered to improve the effectiveness of treatment in the patients enrolled in the study. We also found that most patients required other treatment modalities to control psoriasis during the period of etanercept treatment.